In vitro and in vivo plasmalogen replacement evaluations in rhizomelic chrondrodysplasia punctata and Pelizaeus-Merzbacher disease using PPI-1011, an ether lipid plasmalogen precursor

BACKGROUND: Childhood peroxisomal disorders and leukodystrophies are devastating diseases characterized by dysfunctional lipid metabolism. Plasmalogens (ether glycerophosphoethanolamine lipids) are decreased in these genetic disorders. The biosynthesis of plasmalogens is initiated in peroxisomes but...

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Detalhes bibliográficos
Main Authors: Wood, Paul L, Khan, M Amin, Smith, Tara, Ehrmantraut, Greg, Jin, Wei, Cui, Wei, Braverman, Nancy E, Goodenowe, Dayan B
Formato: Artigo
Idioma:Inglês
Publicado em: BioMed Central 2011
Assuntos:
Research
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC3238230/
https://ncbi.nlm.nih.gov/pubmed/22008564
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1186/1476-511X-10-182
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